Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe.

Elena Cardenal-Muñoz, Stéphane Auvin, Vicente Villanueva, J Helen Cross, Sameer M Zuberi, Lieven Lagae, José Ángel Aibar

Journal: Epilepsia open 2022;7(1):11-26

PMID: 34882995

Abstract

Dravet syndrome (DS) is a severe, rare, and complex developmental and epileptic encephalopathy affecting 1 in 16 000 live births and characterized by a drug-resistant epilepsy, cognitive, psychomotor, and language impairment, and behavioral disorders. Evidence suggests that optimal treatment of seizures in DS may improve outcomes, even though neurodevelopmental impairments are the likely result of both the underlying genetic variant and the epilepsy. We present an updated guideline for DS diagnosis and treatment, taking into consideration care of the adult patient and nonpharmaceutical therapeutic options for this disease. This up-to-date guideline, which is based on an extensive review of the literature and culminates with a new treatment algorithm for DS, is a European consensus developed through a survey involving 29 European clinical experts in DS. This guideline will serve professionals in their clinical practice and, as a consequence, will benefit DS patients and their families.

© 2021 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.

Address: Dravet Syndrome Foundation Spain, Member of the EpiCARE ePAG Group, Madrid, Spain.; APHP. Service de Neurologie Pédiatrique, Hôpital Robert Debré, Paris, France.; INSERM NeuroDiderot, Université de Paris, Paris, France.; Institut Universitaire de France (IUF), Paris, France.; Refractory Epilepsy Unit, Hospital Universitario y Politécnico La Fe, Member of the ERN EpiCARE, Valencia, Spain.; Department of Developmental Neurosciences, UCL NIHR BRC Great Ormond Street Institute of Child Health, London, UK.; Department of Neurology, Great Ormond Street Hospital for Children, Member of the ERN EpiCARE, London, UK.; Paediatric Neurosciences Research Group, Royal Hospital for Children, Member of the ERN EpiCARE, Glasgow, UK.; Institute of Health & Wellbeing, University of Glasgow, Glasgow, UK.; Department of Development and Regeneration, KU Leuven, Member of the ERN EpiCARE, Leuven, Belgium.
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